Chamberlain, Paul D.; Sweeney, Adam R.; Shetlar, Debra J.; Yen, Michael T.
A 69-year-old woman presented with 12 months of progressively worsening painless proptosis and a firm mass protruding from the left superior orbit. She had a history of enucleation for a traumatic blind painful eye 3 years prior with implantation of a porous polyethylene implant. There was no history of trauma following the enucleation, and she was not on any anticoagulant medications. Axial MRI revealed a large, circumscribed mass consisting of hyperintense complex fluid on T2-weighted imaging (Fig. 1A) with enhancing capsule on sagittal postcontrast T1 imaging (Fig. 1B) surrounding the implant and displacing it posteriorly and inferiorly. Intraoperatively, when the capsule of the mass was entered, an abundance of viscous yellow-colored fluid was expressed. Within this capsule, the porous polyethylene implant was floating freely without any integration of blood vessels, connective tissue, or muscle. The capsule containing the implant was excised in its entirety, and a silicone sphere implant was placed in the orbit. Histology of the excised capsule showed benign epithelium with dense fibrous tissue and numerous cholesterol clefts (asterisk) admixed with red blood cells, lipid laden histiocytes, and chronic inflammation (Fig. 2, hematoxylin and eosin, ×200). These findings are consistent with changes seen with chronic hemorrhage. While porous polyethylene implants are typically used to allow for fibrovascular ingrowth, chronic or late bleeding in the orbit may prevent or disrupt such integration from developing. Retained intracapsular hemorrhage may mimic an orbital mass, and if symptomatic, may necessitate removal of the implant, capsule, and hemorrhage. Definitive diagnosis requires tissue for pathologic evaluation.
Shikha Taneja, Tariq Aldoais & Swathi Kaliki
Purpose: To evaluate the outcome of primary orbital polymethylmethacrylate (PMMA) implant following the primary enucleation for retinoblastoma.
Methods: Retrospective study of 321 retinoblastoma patients who underwent unilateral enucleation and PMMA implant for retinoblastoma by myoconjunctival technique. Outcome measures included implant centration and extrusion.
Results: The mean age at the time of enucleation of patients with retinoblastoma was 35 months (median, 30 months; range, <1 to 449 months). After primary enucleation, primary orbital PMMA implant was used in all cases. The mean diameter of implant was 18 mm (median, 18 mm; range, 12–20 mm) and the mean horizontal diameter of the socket conformer was 24 mm (median, 24 mm; range, 18 mm–26 mm). Post-enucleation and implant, seven (2%) patients underwent orbital external beam radiotherapy owing to microscopic extrascleral tumor extension or tumor infiltration of optic nerve transection. Over a mean follow-up period of 40 months (median, 34 months; range, 4–129 months), implant migration was noted in 28 (9%) patients, implant extrusion in 9 (3%), and implant exposure in 5 (2%), and contracted socket in 5 (2%) patients including grade 1 contraction in 3 (1%), grade 2 in 1 (<1%), and grade 4 in 1 (<1%) patient. Implant exchange for an improved prosthesis fit was performed in 4 (1%) cases. Stable customized ocular prosthesis was achieved in all but one patient.
Conclusion: Primary orbital PMMA implant following primary enucleation for retinoblastoma is associated with minimal complications and provides acceptable cosmetic outcomes.
Zhu, Yanling; Li, Zuohong; Chen, Wenshi; Fan, Peiting; Yang, Shiying; Liu, Xuehua; Guo, Wenjun; Gan, Xiaoliang
To prospectively explore the incidence and risk factors of moderate to severe pain after primary and secondary orbital implantation following evisceration or enucleation surgery.
One hundred eighteen patients under general anesthesia for orbital implantation were enrolled in this study. In 91 patients, primary orbital implantation followed evisceration, and in 27 patients, the implantation was secondary after previous evisceration or enucleation surgery. Medical interventions for all participants were followed by standardized surgical, anesthetic, and analgesic protocols. Postoperative pain (POP) intensity was quantified by an 11-point numerical rating scale within 72 hours after the surgery, numerical rating scale ≥4 was considered moderate to severe POP. Multivariate logistic regression was utilized to identify the risk factors related to the development of POP.
Thirty-five patients (29.7%) displayed moderate to severe POP, particularly within 6 to 24 hours after surgery, which peaked at 24 hours. Of these patients, 26 patients who were unable to tolerate the pain received additional doses of analgesics during in-hospital stay. Logistic regression model revealed that preoperative anxiety (odds ratios = 4.890; p = 0.002), congenital microphthalmia (odds ratios = 14.602; p = 0.038), and surgical time longer than 60 minutes (odds ratios = 5.586; p = 0.001) were significantly associated with moderate to severe POP after orbital implantation.
Orbital implantation after evisceration or enucleation surgery is likely to cause moderate to severe pain intensity in the early postoperative period. Preoperative anxiety, prolonged surgical time, and congenital microphthalmia were the risk factors.
Effy Ojuok, Aditya Uppuluri, Paul D. Langer, Marco A. Zarbin, Loka Thangamathesvaran & Neelakshi Bhagat
Trauma is the leading cause of enucleations in the USA. Current information regarding open globe injuries (OGI) is based mainly on data from individual tertiary care centers across the country which might skew the findings towards the population served by these level-one trauma centers. The aim of this study is to evaluate the demographics, characteristics, and risk factors of traumatic enucleations in a large data sample.
Descriptive cross-sectional observational study using the National Inpatient Sample (NIS) Database from 2002 to 2013. Inpatients with traumatic enucleations were identified using ICD-9 codes. Chi-square and logistic regression analyses were used to identify differences between the enucleated and non-enucleated cohorts and to evaluate the predictive factors of enucleation in OGIs.
Enucleations were performed in 3020 (6.2%) of 48,563 OGIs identified. The average age in the enucleated cohort for males vs. females was 44.7 vs. 62.2 years. In the USA, the highest number of traumatic enucleations occurred in the 21–40 group (41.8%) and the fewest in the 80+ age group (11.8%). The risk of enucleation decreased across the age groups significantly. Compared with the 21–40 age group, the risk of undergoing enucleation was 15% lower in patients 41 to 60 years of age, 35% in patients 61 to 80, and 40% lower in patients over 80. In total, 5.1% OGIs in women and 6.7% of OGIs in men were enucleated. The risk of enucleation was 29% higher in men than in women. The highest absolute number of enucleations was seen in Whites. Compared with Whites, Blacks had a 63% higher risk of enucleation following an OGI. OGIs with rupture-type injury, endophthalmitis, or phthisis were significantly higher odds to be enucleated.
The risk of enucleation following traumatic OGI significantly increased for patients who were in the 21–40 age group, of Black race, or of male gender; the risk also increased if the injury was a rupture-type or associated with endophthalmitis or phthisis. The risk of depression was 75% higher in enucleated patients versus non-enucleated patients.
Hind M. Alkatan, Saad A. Al-Dahmash, Saleh A. Almesfer, Faisal S. AlQahtani & Azza M. Y. Maktabi
The management of bilateral advanced retinoblastoma (RB) cases is challenging with attempts to use neoadjuvant therapy salvaging of one of the globes. Our aim in this study was to demonstrate the effect of this primary therapy on the histopathological features and risk factors in secondary enucleated compared to primarily enucleated globes with groups D and E RB.
We retrospectively reviewed all enucleated globes with advanced RB received in the pathology laboratories over a period of 5 years. Patients were divided into two groups: one with primary enucleations and another with at least one secondary enucleated globe, and their demographic and clinical data were analyzed. The enucleated globes in the two groups were analyzed to compare the general histopathological features including tumor seeding, size, differentiation, growth pattern, mitotic figures, and focality. More importantly, high-risk features: choroidal invasion, optic nerve (ON) invasion, iris/anterior chamber invasion, ciliary body invasion, and scleral and extra-scleral extension, as well as the pathological classification of the tumor (pT) according to the American Joint Committee on Cancer 7th edition were also compared between the two groups.
We had a total of 106 enucleated globes (78 primary and 28 secondary enucleations) from 99 patients with advanced RB (73 patients with primarily and 26 with secondarily enucleated globes). Demographic and clinical profiles of patients were similar in both, but the mean interval from presentation to enucleation was significantly longer in the secondary enucleations (P = 0.015). Rare/occasional mitotic figures were observed in secondary enucleations using multivariate analysis (P = 0.003). Primarily enucleated globes had higher risk of tumor seeding (P = 0.020), post-laminar/surgical margin ON invasion (P = 0.001), and massive choroidal invasion (P = 0.028). Half of the secondary enucleated globes had tumors confined to the globes without invasion (pT1) and statistically significant lower tumor classifications (pT1 or pT2a) compared to primary enucleations (P =0.001). However, 18% of the secondarily enucleated globes in 3 patients had unfavorable outcome with RB-related mortality after a period of 1–4 years.
Secondary enucleated globes with advanced RB show favorable histopathological findings mainly less mitosis. These eyes have significantly lower chance for harboring choroidal and ON invasion, thus mostly classified as pT1 or pT2a when compared to primarily enucleated globes. The decision for secondary enucleation was observed to be significantly delayed (8.0 months ± 9.8). Prompt decision for needed enucleation based on the response to primary treatment and careful histopathological examination of enucleated globes are essential to prevent disease-related mortality.
Swathi Kaliki, Shweta Gupta, George Ramappa, Ashik Mohamed & Dilip K. Mishra
To study the high-risk histopathology features of retinoblastoma based on age at primary enucleation.
Retrospective study of 616 patients.
The mean age at presentation and primary enucleation for retinoblastoma was 34 months (median, 28 months; range, <1–455 months). Of these cases, 128 (21%) were aged ≤1 year, 149 (24%) were in the age group of 1–2 years, 117 (19%) in 2–3 years, 104 (17%) in 3–4 years, and 118 (19%) were >4 years of age at the time of enucleation. Bilateral retinoblastoma (34%; p < 0.0001) and buphthalmos (20%; p < 0.0001) were more common in children ≤1 year of age. Anterior chamber pseudohypopyon (15%; p < 0.0001) and vitreous seeds (53%; p < 0.0001) were more common in children aged >4 years. Based on 8th edition American Joint Committee on Cancer staging system, pT3 was less common in children ≤1 year of age (13%; p < 0.001). Based on histopathology, 38% patients had high-risk features including 24% children aged ≤1 year, 42% in the age group of 1–2 years, 34% in 2–3 years age group, 45% in 3–4 years age group, and 48% patients were >4 years of age. Post-laminar optic nerve infiltration (6%; p = 0.02) and massive choroidal infiltration (9%; p = 0.04) was least common in children ≤1 year of age. Over a mean follow-up period of 52 months (median, 36 months; range, <1–218 months), systemic metastasis and death occurred in 9% patients despite adjuvant systemic chemotherapy.
The predominant high-risk histopathology feature of retinoblastoma varies with age at primary enucleation.
Eva Maria Biewald, Norbert Bornfeld, Klaus A Metz, Sabrina Schlüter, Tobias Kiefer, Alexander Radbruch, Sophia Göricke, Selma Sirin, Petra Ketteler, Nikolaos E Bechrakis
Background To demonstrate histopathological findings in retinoblastoma eyes enucleated after intra-arterial chemotherapy (IAC) with special emphasis on vascular toxicity and local tumour control.
Methods Retrospective study with a consecutive series of 23 retinoblastoma eyes enucleated after IAC where histopathological work-up was available.
Results From November 2010 to June 2019 23 eyes were enucleated after the attempt of eye salvaging therapy with IAC using melphalan. IAC was the first line treatment in nine and salvage treatment in 14 eyes. Doses of melphalan ranged from 3 to 7.5 mg, whereby a strict protocol with age-appropriate dosage was not used until 2015. The mean number of treatment cycles was 1.8. The main indications for enucleation were poor treatment response or tumour progression in 14 eyes, severe vascular complications in five eyes and a total exudative retinal detachment with amaurosis in the remaining four eyes. We found active disease in 15 eyes with an indication for adjuvant chemotherapy due to high risk factors for metastases in four eyes. To date none of these patients developed metastatic disease. Concerning vascular toxicity, we detected a central retinal artery occlusion in three eyes, severe vasculitis in another three, ischaemic outer retina atrophy and choroidal ischaemia in seven eyes with one eye developing a severe proliferative retinopathy.
Conclusion IAC is a highly effective treatment option for advanced retinoblastoma, but the described potential risks should be kept in mind. These include severe vascular complications, as well as the possibility of persisting vital tumour cells fulfilling high-risk criteria for adjuvant chemotherapy.
Hind Manaa Alkatan, Faisal Saeed AlQahtani , Azza MY Maktabi
We aim to study certain histopathological characteristics of the retinoblastoma (RB) tumors in globes with clinically advanced RB, which can be correlated with the high-risk features including: tumor differentiation, growth pattern and focality. We also aim to reclassify the tumor pathologically in the analyzed cases according to the American Joint Committee on Cancer (AJCC) 8th edition in an attempt to compare the validity of this newest classification.
Retrospective study of patients diagnosed with clinical RB of groups D and E during the period: January 2013 to December 2017 at King Khaled Eye Specialist Hospital (KKESH) and King Abdulaziz University Hospital (KAUH). Charts were reviewed for demographic and basic clinical data. Histopathological features (tumor differentiation, growth pattern, focality, seeding, and presence of choroidal invasion (focal versus massive), level of optic nerve (ON) invasion, anterior chamber invasion, scleral and extra-scleral extension, and finally the documented pathological tumors (pT) classification based on the AJCC 7th edition were collected.
We included 146 eyes with advanced retinoblastoma (groups D and E) from 104 patients. Gender distribution was almost equal with 54 males. Median age was 12 months (range 1–96 months), and a mean age was 17.1 ± 15.1 months. No family history was found in the majority (94.2%). Clinically, the most common presenting symptoms: leukocoria (73.3%), squint (33.6%) and least commonly proptosis (2.7%). A total of 106 enucleation specimens were reviewed. Degree of RB differentiation was: well differentiated (18%), moderate (30%), poor (35%), and undifferentiated (17%). The most common high-risk features were ON invasion (68%) with the majority being prelaminar in nature, choroidal invasion (45%) with more than half being massive, iris/trabecular meshwork (TM)/Schlemm’s canal invasion (8.5%), and then intra-scleral/extra-scleral extension (5%). Less tumor differentiation doubles the relative risk of massive choroidal invasion (with 95% CI) with a statistically significant P value (P = 0.030). Endophytic RB was associated with vitreous seeding, while exophytic tumors were associated with subretinal seeding (P = 0.001) each. Ten cases with combined ON invasion (pre-laminar) and focal choroidal invasion were reclassified pT2a in the AJCC 8th edition instead of pT2b in the older 7th edition.
Our demographic and basic clinical data for advanced RB are comparable to other similar reports. The tumor growth pattern correlates well with the type of seeding observed in enucleated globes with RB. Less tumor differentiation is a relative risk of massive choroidal invasion. It is advocated to implement the AJCC 8th edition by ocular pathologists worldwide aiming to histopathologically classify the RB tumor in cases for selective adjuvant chemotherapy.
Godbout, Erin E.; Lau, Kevin W.; Adam, Alejandro P.; Wladis, Edward J.
To determine if calcitonin gene-related peptide (CGRP) is overexpressed in blind, painful eyes.
This was a retrospective cross-sectional randomized control study conducted at a tertiary level hospital. Eight specimens of eyes enucleated by a single surgeon were included in the study. The control group patients underwent exenteration for cutaneous malignancy without intraocular involvement, while the case group was enucleated for management of blind, painful eye. Each eye was stained using immunohistochemistry for the expression of CGRP. Expression of CGRP was examined by counting the number of cells staining positive for CGRP in 5 consecutive ×40 fields in the choroid, iris, and cornea of each specimen.
The mean number of cells staining positively for CGRP in the choroid of blind, painful eye group was 45.5 (26.3) versus 5.5 (7.7) in nonpainful enucleated eyes. This difference was found to be statistically significant (p = 0.016). Comparison of iris and cornea did not reach statistical significance. Expression of CGRP was compared using a paired t test.
This study demonstrates that CGRP is overexpressed in the choroid of enucleated blind, painful eyes. Modulation of this protein may represent a meaningful, nonsurgical therapeutic strategy to addressing the blind, painful eye.